Stefek Bryan P, Imundo Jason R, Clark Joseph B
Tex Heart Inst J. 2019 Jun 1;46(3):225-228. doi: 10.14503/THIJ-17-6521. eCollection 2019 Jun.
Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient's case, as well as other considerations in this combination of congenital defects.
右冠状动脉起源于肺动脉，这是一种罕见的先天性心脏缺陷，在婴儿期通常无法诊断。另一方面，特纳综合征通常能早期诊断，且典型地与二叶式主动脉瓣和主动脉缩窄相关。患有特纳综合征的个体发生冠状动脉异常的风险也会增加。我们报告一例一周大的新生儿，其右冠状动脉起源于肺动脉，同时还患有特纳综合征、动脉导管未闭、主动脉弓横部发育不全和心室功能受损。通过动脉导管进行前列腺素治疗增加了患者的心肌灌注。矫正手术后四个月，她情况良好。我们讨论了该患者病例中的再灌注现象，以及这种先天性缺陷组合中的其他注意事项。
